Chia-Feng Yang

Spirometric pulmonary function parameters of healthy Chinese children aged 3–6 years in Taiwan†

Spirometry is a well‐known technique for evaluating pulmonary function, but few studies have focused on preschool children. The aim of this study was to determine reference values of forced spirometric parameters in young Chinese children, aged 3–6 years, in Taiwan. Spirometric measurements were performed at day care centers by experienced pediatricians. Of 248 children without a history of chronic respiratory illness, at least two valid spirometric attempts were obtained from 214 children (109 boys and 105 girls; age: 36–83 [mean = 61] months; height: 90–131 [mean = 111] cm). Values of forced expiratory volume in 1 sec (FEV1) and 0.5 sec (FEV0.5), forced vital capacity (FVC), peak expiratory flow rate (PEF), forced expiratory between 25% and 75% FVC (FEF25–75), and forced expiratory flow rate at 25%, 50%, and 75% of FVC (FEF25, FEF50, and FEF75) were derived and analyzed. There were significant positive correlations between study parameters and body height, body weight, and age. Height was the most consistently correlated measurement in both boys and girls. Although boys tended to have higher spirometric values than girls, we found significant differences only in FVC and FEV1 between boys and girls aged 6 years. The regression equations of each parameter were obtained. In conclusion, spirometric pulmonary function tests are feasible in 3‐ to 6‐year‐old children. The obtained values and regression equations provide a reference for Chinese preschool children and may be of value in evaluating pulmonary function of children with respiratory problems in this age group. Pediatr Pulmonol. 2009; 44:676–682.

Esophageal Atresia with Tracheoesophageal Fistula: Ten Years of Experience in an Institute

Background: Esophageal atresia (EA), tracheoesophageal fistula (TEF), or both is a complicated problem. The purpose of this study was to evaluate the outcomes and postoperative complications in patients with EA/TEF who were admitted to our hospital. Methods: In total, 15 patients were enrolled from 1994 to 2003, including 8 males and 7 females. Patient demographics, associated anomalies, and outcomes were analyzed. Results: The most common variant was EA with a distal TEF (type C), which occurred in 12 patients (80%). The latter had associated congenital anomalies, and cardiac anomalies were the most frequent, occurring in 8 patients (53.3%). Of the 6 cases who had life‐threatening anomalies, 4 (66.7%) died, and of the 9 cases who had no life‐threatening anomalies, 2 (22.2%) died. Tracheomalacia and/or stenosis were diagnosed in 8 patients (66.7%) postoperatively. Though 3 of the 4 cases who suffered from dying spell received intratracheal stent implantation, 2 cases still died. Conclusion: The survival rate of the patients with EA/TEF is influenced mainly by associated life‐threatening anomalies. TMS combined with a history of dying spell may be the major fatal complication.

Flexible bronchoscopy as a valuable diagnostic and therapeutic tool in pediatric intensive care patients: A report on 5 years of experience†

To evaluate the clinical role of flexible bronchoscopy (FB) in pediatric and neonatal intensive care units (ICUs).

Hyperbilirubinemia with urinary tract infection in infants younger than eight weeks old

Background: Hyperbilirubinemia is one of the most common causes for hospital admission in neonatal infants. Previous studies have found that jaundice may be one of the initial symptoms related to urinary tract infection (UTI) in infants. This study is to evaluate the incidence and related factors of neonatal infants with the initial presentation of hyperbilirubinemia and final diagnosis of UTI in a tertiary teaching hospital. Methods: We retrospectively investigated the medical records of admitted infants younger than 8 weeks old with hyperbilirubinemia between January and December 2008. The jaundiced infants having tests of urinalysis were enrolled into our study and grouped into UTI or no UTI group according to the findings of urinary culture. Results: A total of 217 neonatal jaundiced infants were enrolled. Among them, 12 cases (5.5%) were grouped into the UTI group, and the most common cultured bacterium from their urine was Escherichia coli. There was no significant difference in the babies’ birth weight, maternal conditions, or total bilirubin levels between the two groups. There was also no significant difference between the two groups in their admission age (9.7 ± 13.5 days vs. 6.1 ± 6.7 days in UTI and no UTI groups, respectively) or the ratio of outpatients (50% vs. 25% in UTI and no UTI groups, respectively) (p > 0.05). The cases of UTI group had significantly lower hemoglobin (15.2 ± 2.7 g/dL vs. 17.2 ± 2.3 g/dL, respectively) and higher formula feeding rate (8.3% vs. 2.9%, respectively) than the no UTI group (p < 0.05). Conclusion: The incidence of UTI in the admitted infants with hyperbilirubinemia was as high as approximately 5.5%. The most common cultured bacterium in urine was E coli. Therefore, performing urinary tests to exclude the possibility of coincidental UTI may be necessary for admitted jaundiced infants younger than 8 weeks old.

The epidemiology and prognostic factors of mortality in critically ill children with acute kidney injury in Taiwan

The incidence of acute kidney injury (AKI) in critically ill children varies among countries. Here we used claims data from the Taiwanese National Health Insurance program from 2006 to 2010 to investigate the epidemiological features and identify factors that predispose individuals to developing AKI and mortality in critically ill children with AKI. Of 60,338 children in this nationwide cohort, AKI was identified in 850, yielding an average incidence rate of 1.4%. Significant independent risk factors for AKI were the use of extracorporeal membrane oxygenation, mechanical ventilation or vasopressors, intrinsic renal diseases, sepsis, and age more than 1 year. Overall, of the AKI cases, 46.5% were due to sepsis, 36.1% underwent renal replacement therapy, and the mortality rate was 44.2%. Multivariate analysis showed that the use of vasopressors, mechanical ventilation, and hemato-oncological disorders were independent predictors of mortality in AKI patients. Thirty-two of the 474 patients who survived had progression to chronic kidney disease or end-stage renal disease. Thus, although not common, AKI in critically ill children still has a high mortality rate associated with a variety of factors. Long-term close follow-up to prevent progressive chronic kidney disease in survivors of critical illnesses with AKI is mandatory.

Very Early Treatment for Infantile-Onset Pompe Disease Contributes to Better Outcomes

OBJECTIVE To evaluate whether very early treatment in our patients would result in better clinical outcomes and to compare these data with other infantile-onset Pompe disease (IOPD) cohort studies. METHODS In this nationwide program, 669,797 newborns were screened for Pompe disease. We diagnosed IOPD in 14 of these newborns, and all were treated and followed in our hospital. RESULTS After 2010, the mean age at first enzyme-replacement therapy (ERT) was 11.92 days. Our patients had better biological, physical, and developmental outcomes and lower anti-rh acid α-glucosidase antibodies after 2 years of treatment, even compared with one group that began ERT just 10 days later than our cohort. No patient had a hearing disorder or abnormal vision. The mean age for independent walking was 11.6 ± 1.3 months, the same age as normal children. CONCLUSIONS ERT for patients with IOPD should be initiated as early as possible before irreversible damage occurs. Our results indicate that early identification of patients with IOPD allows for the very early initiation of ERT. Starting ERT even a few days earlier may lead to better patient outcomes.

Pulmonary Function Changes in Children After Transcatheter Closure of Atrial Septal Defect

This study was performed to assess changes in pulmonary function test (PFT) and pulmonary outcome after transcatheter closure of atrial septal defect (ASD) in pediatric patients. A total 55 pediatric patients undergoing transcatheter ASD closure received PFT at baseline (day before ASD closure), and at 3 days and 6 months after procedure. Forced vital capacity (FVC), forced expired volume in 1 sec (FEV1), FEV1 to FVC ratio (FEV1/FVC), peak expiratory flow (PEF), and mean forced expiratory flow during the middle half of FVC (FEF25–75) were measured. Individually, subjects were classified by spirometry as normal, obstructive or restrictive, to evaluate the effect of transcatheter closure on pulmonary outcome. These 55 children had significantly reduced mean PEF and FEF25–75 (84 ± 24%, P = 0.040 and 76 ± 22%, P = 0.010, respectively) at baseline, with FEF25–75 reduced significantly at 3 days and 6 months (78 ± 24%, P = 0.010 and 81 ± 24%, P = 0.040, respectively) after transcatheter closure. Six months after transcatheter closure of ASD, significant improvement was observed in mean FVC (94 ± 19% vs. 98 ± 15%, P = 0.034) and FEV1 (90 ± 20% vs. 96 ± 19%, P = 0.008). Assessed individually, better pulmonary outcome was found in patients without pulmonary hypertension (PH) (χ2 = 8.333, P = 0.044). PFT disturbance was observed in significant flow limitation in the peripheral airway of ASD patients. Improved PFT was found after transcatheter closure and better pulmonary outcome was observed in patients without PH. ASD children need monitoring pulmonary function and should receive transcatheter closure before PH develops. Pediatr Pulmonol. 2009; 44:1025–1032. ©2009 Wiley‐Liss, Inc.

Pediatric obstructive fibrinous tracheal pseudomembrane--characteristics and management with flexible bronchoscopy.

OBJECTIVE To evaluate the incidence, characteristics, flexible bronchoscopy (FB) findings, interventions and outcome of pediatric obstructive fibrinous tracheal pseudomembrane (OFTP) in our pediatric and neonatal intensive care units (ICUs). PATIENTS AND METHODS This is a retrospective study of medical and FB video records in a single tertiary university-affiliated teaching hospital over a ten-year period. Data was collected from patients who were admitted and extubated of endotracheal tube in the ICUs with a FB diagnosis of an OFTP-like lesion. The associated medical information, FB interventions and video records were reviewed and analyzed. RESULTS Eight patients with OFTP were enrolled, with an incidence rate of 1.48% in the postextubation respiratory distress patients. Mean age was 32 ± 32 months (range, 2 months to 13 years); mean body weight was 13.7 ± 8.1 kg (range, 4.3-45 kg); mean intubation period was 37.6 ± 12.3h; mean time for symptoms to develop after extubation was 3.6 ± 1.4h. Symptoms lasted for 20.8 ± 20.3h before FB examination. All patients were accurately diagnosed with OFTP at the first postextubation FB examination and revealed various morphologies. The estimated cross-sectional tracheal lumen was reduced by 70-90% and the mean length of lesion was 18.1 ± 5.2mm (range, 10-30 mm). All OFTP were successfully ablated immediately after the diagnosis in one FB session by using various techniques and without any complication. Total duration for both diagnostic and interventional FB was 19.4 ± 2.5 min. No recurrence was noted thereafter. CONCLUSIONS OFTP should always be considered in the event of postextubation respiratory distress, especially in the pediatric and neonatal ICUs. Early diagnosis and effective ablation can be achieved with aid of FB.

Mass Spectrometry but Not Fluorimetry Distinguishes Affected and Pseudodeficiency Patients in Newborn Screening for Pompe Disease

BACKGROUND Deficiency of the lysosomal enzyme acid α-glucosidase (GAA) causes Pompe disease. Newborn screening for Pompe disease is ongoing, and improved methods for distinguishing affected patients from those with pseudodeficiency, especially in the Asian population, would substantially reduce the number of patient referrals for clinical follow-up. METHODS We measured the enzymatic activity of GAA in dried blood spots on newborn screening cards (DBS) using a tandem mass spectrometry (MS/MS) assay. The assay displayed a relatively large analytical range compared to the fluorimetric assay with 4-methylumbelliferyl-α-glucoside. DBS from newborns confirmed to have infantile-onset Pompe disease (IOPD, n = 11) or late-onset Pompe disease (LOPD) (n = 12) and those from patients bearing pseudodeficiency alleles with or without Pompe mutations, or Pompe disease carriers (n = 230) were studied. RESULTS With use of the MS/MS GAA assay in DBS, 96% of the pseudodeficiency newborns and all of the Pompe disease carriers were well separated from the IOPD and LOPD newborns. The fluorimetric assay separated <10% of the pseudodeficiencies from the IOPD/LOPD group. CONCLUSIONS The relatively large analytical range MS/MS GAA assay but not the fluorimetric assay in DBS provides a robust approach to reduce the number of referrals and should dramatically facilitate newborn screening of Pompe disease.

Cranial ultrasonographic findings in healthy full-term neonates: a retrospective review.

Background: Ultrasonography is a non‐invasive diagnostic technique, and it has been used to detect intracranial lesions in neonates for a long time. Correspondingly, screening tests using cranial ultrasonography have been applied for early detection of intracranial lesions in full‐term neonates during the past decade. Methods: We retrospectively reviewed the findings of cranial ultrasonographic screening tests in healthy full‐term neonates between September 2004 and August 2009. The ultrasonographic findings were divided into the following categories: (a) nonsignificant (NS) group, including normal and normal variations, (b) minor anomaly group, including tiny cystic lesions, mild hemorrhage, or mild ventricular anomaly, and (c) major anomaly group, including significant anomaly of any intracranial pathology. The participants with major anomalies were further reviewed, and the following medical records of all enrolled patients were reviewed until they were 24 months of age. Results: There were a total of 3186 neonates who received cranial ultrasonographic screening examination during the 5‐year period, and most of them (2982 cases, 93.6%) were assigned to the NS group. The most common normal variation was the presence of cavum septum pellucidum (1979 cases, 62.1%). Minor anomalies were found in 202 (6.3%) neonates, including 119 (3.7%) neonates with tiny cysts, and 59 (1.9%) neonates with mild intraventricular hemorrhage. Major anomalies were found in two (0.06%) neonates, including obstructive hydrocephalus and agenesis of the corpus callosum. Two other infants (0.06%) initially presented with minor anomaly or normal variation, but they were diagnosed as Moyamoya disease and neonatal seizure some months later. Conclusion: The incidence of minor and major anomalies detected by cranial ultrasonographic screening examinations in healthy full‐term neonates is 6.3% and 0.06%, respectively. Thus, cranial ultrasonographic screening testing may play a role in the early diagnosis of intracranial anomalies of otherwise healthy neonates. However, this examination cannot exclude or detect all cranial abnormalities, including many potential neurologic diseases of neonates, so continuing clinical diligence is still important for all infants.